METHODS: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined.
RESULTS: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort.
CONCLUSIONS: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit.
方法:在单个中心(2000年1月至2020年6月)对接受KP的患者进行了回顾性审查。检查临床结果。
结果:在12例患者中,有11例进行了初步缓解。KP时的年龄和体重分别为8.25个月(6.4-9.7)和7.7kg(6.5-8.6)。重症监护病房住院3.16天(1-12天),总住院时间为9.5天(3-22天).有一次计划外的手术,队列中没有死亡率。放电氧饱和度为88%(80%-98%)。7例患者发生了肺动静脉畸形(PAVMs),其中6例进行了肝静脉掺入(HVI)。PAVM发展的间隔时间为42.3个月(16-121)。12例患者中有4例存在顺行肺血流(PBF),3例(75%)没有PAVM。中位随访时间为10年(1.5-22年),队列12例中有11例(91.67%)生存。
结论:KP可以在比以前报道的更年轻的年龄进行,并具有足够的早期和晚期结果。大多数患者将继续发展PAVM并需要HVI,但留下一些顺行PBF的患者可能具有保护性,但需要进一步调查以显示明确的益处。